Similarly, mice with a knockout of the C-terminal spectrin repeat region of nesprin-1 which resulted in ablation of most isoforms of nesprin-1 showed defects in positioning and anchorage of nuclei in skeletal muscle, but had normal heart function and did not display a phenotype similar to EDMD [36]. This evidence concerns the gene SYNE1 and Emery-Dreifuss muscular dystrophy.